Abstract:
:Whipple disease (WD) is a rare multisystemic infection with a protean clinical presentation. The central nervous system (CNS) is involved in 3 situations: CNS involvement in classic WD, CNS relapse in previously treated WD, and isolated CNS infection. We retrospectively analyzed clinical features, diagnostic workup, brain imaging, cerebrospinal fluid (CSF) study, treatment, and follow-up data in 18 patients with WD and CNS infection. Ten men and 8 women were included with a median age at diagnosis of 47 years (range, 30-56 yr). The median follow-up duration was 6 years (range, 1-19 yr). As categorized in the 3 subgroups, 11 patients had classic WD with CNS involvement, 4 had an isolated CNS infection, and 3 had a neurologic relapse of previously treated WD. CNS involvement occurred during prolonged trimethoprim-sulfamethoxazole (TMP-SMX) treatment in 1 patient with classic WD. The neurologic symptoms were various and always intermingled, as follows: confusion or coma (17%) related to meningo-encephalitis or status epilepticus; delirium (17%); cognitive impairment (61%) including memory loss and attention defects or typical frontal lobe syndrome; hypersomnia (17%); abnormal movements (myoclonus, choreiform movements, oculomasticatory myorhythmia) (39%); cerebellar ataxia (11%); upper motor neuron (44%) or extrapyramidal symptoms (33%); and ophthalmoplegia (17%) in conjunction or not with progressive supranuclear palsy. No specific pattern was correlated with any subgroup. Brain magnetic resonance imaging (MRI) revealed a unique focal lesion (35%), mostly as a tumorlike brain lesion, or multifocal lesions (23%) involving the medial temporal lobe, midbrain, hypothalamus, and thalamus. Periventricular diffuse leukopathy (6%), diffuse cortical atrophy (18%), and pachymeningitis (12%) were observed. The spinal cord was involved in 2 cases. MRI showed ischemic sequelae at diagnosis or during follow-up in 4 patients. Brain MRI was normal despite neurologic symptoms in 3 cases. CSF cytology was normal in 62% of patients, whereas Tropheryma whipplei polymerase chain reaction (PCR) analysis was positive in 92% of cases with tested CSF. Periodic acid-Schiff (PAS)-positive cells were identified in cerebral biopsies of 4 patients. All patients were treated with antimicrobial therapy for a mean duration of 2 years (range, 1-7 yr) with either oral monotherapy (TMP-SMX, doxycycline, third-generation cephalosporins) or a combination of antibiotics that sometimes followed parenteral treatment with beta-lactams and aminoglycosides. Eight patients also received hydroxychloroquine. At the end of follow-up, the clinical outcome was favorable in 14 patients (78%), with mild to moderate sequelae in 9. Thirteen patients (72%) had stopped treatment for an average time of 4 years (range, 0.7-14 yr). Four patients had clinical worsening despite antimicrobial therapy; 2 of those died following diffuse encephalitis (n = 1) and lung infection (n = 1). In conclusion, the neurologic manifestations of WD are diverse and may mimic almost any neurologic condition. Brain involvement may occur during or after TMP-SMX treatment. CSF T. whipplei PCR analysis is a major tool for diagnosis and may be positive in the absence of meningitis. Immune reconstitution syndrome may occur in the early months of treatment. Late prognosis may be better than previously reported, as a consequence of earlier diagnosis and a better use of antimicrobial therapy, including hydroxychloroquine and doxycycline combination.
journal_name
Medicine (Baltimore)journal_title
Medicineauthors
Compain C,Sacre K,Puéchal X,Klein I,Vital-Durand D,Houeto JL,De Broucker T,Raoult D,Papo Tdoi
10.1097/MD.0000000000000010subject
Has Abstractpub_date
2013-11-01 00:00:00pages
324-330issue
6eissn
0025-7974issn
1536-5964pii
00005792-201311000-00004journal_volume
92pub_type
杂志文章相关文献
MEDICINE文献大全abstract:BACKGROUND:Nasal surgeries have been applied to obstructive sleep apnea (OSA) patients with nasal obstruction for decades. However, the efficiency of nasal surgery in improving OSA remains controversial.The aim of this study was to identify whether isolated nasal surgery can improve apnea-hypopnea index (AHI). METHODS...
journal_title:Medicine
pub_type: 杂志文章,meta分析,评审
doi:10.1097/MD.0000000000006008
更新日期:2017-02-01 00:00:00
abstract:BACKGROUND:According to the mechanisms of action, combination therapy of anabolic and antiresorptive agents may produce more effect for the treatment of osteoporosis. However, the combination therapy of anabolic agents and bisphosphonates reports no benefit and even reduced the anabolic effects of anabolic agents. This...
journal_title:Medicine
pub_type: 杂志文章,meta分析
doi:10.1097/MD.0000000000009534
更新日期:2017-12-01 00:00:00
abstract:BACKGROUND:This study will assess the effect of high-quality nursing (HQN) on alleviating depression and anxiety (DA) in patients with thyroid cancer (TC) during perioperative period (PPP). METHODS:We will search the following electronic databases (MEDLINE, EMBASE, PsycINFO, Cochrane Library, CINAHL, Web of Science, C...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0000000000023018
更新日期:2020-11-06 00:00:00
abstract::Surgical intervention is an important option for treating lumbar tuberculosis. Previous studies have reported different surgical intervention procedures. To our knowledge, few studies have compared the clinical results of mid-term follow-up of 3 different surgeries in surgical treatment of spinal tuberculosis. This st...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0000000000008574
更新日期:2017-11-01 00:00:00
abstract::The purpose of this study was to examine the relationship between serum ferritin levels and metabolically obese normal weight (MONW) and to determine the appropriate cut-off value of serum ferritin for the prediction of clinical metabolic status in nonobese Korean adults. Data from 9411 participants in the fourth (200...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0000000000002335
更新日期:2015-12-01 00:00:00
abstract::This study aims to evaluate the clinical and radiographic treatment outcomes of comminuted Mason type II radial head fractures, which underwent open reduction and internal fixation (ORIF) using a new implant (mother-child screw, MCS).This study included 16 patients (7 male and 9 female patients; mean age: 40.9 years, ...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0000000000010086
更新日期:2018-03-01 00:00:00
abstract:RATIONALE:Anti-glomerular basement membrane (GBM) disease is a T cell-mediated disease that has a poor prognosis with conventional therapy. We tested rituximab as a primary therapy to reduce anti-GBM antibody produced by B cells. PATIENT CONCERNS:A 53-year old woman with complaints of a fever, headache and abdominal d...
journal_title:Medicine
pub_type: 杂志文章,评审
doi:10.1097/MD.0000000000017801
更新日期:2019-11-01 00:00:00
abstract::The aim of the study was to investigate current situation of postoperative pain management in medical institutions in Shandong Province.A questionnaire was developed on the basis of guidelines of acute pain and pain quality assessment scale. The questionnaire was used to obtain information regarding the nature and sca...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0000000000002690
更新日期:2016-02-01 00:00:00
abstract:OBJECTIVE:The aim of this study was to observe the counter-effect of carbon monoxide-releasing molecule-2 (CORM-2) on lipopolysaccharide (LPS)-suppressed thrombomodulin (TM) and endothelial protein C receptor (EPCR) expressions from human umbilical vein endothelial cell (HUVEC), and to reveal its mechanisms. METHODS:H...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0000000000006978
更新日期:2017-05-01 00:00:00
abstract:RATIONALE:Surgical removal of a sebaceous cyst is usually accomplished through an external incision, which inevitably results in a scar. Herein, we described an intraoral approach for excising sebaceous cysts located in the lip or cheek near lip commissure, to avoid a skin scar. PATIENT CONCERNS:Removal of the cyst bu...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0000000000008803
更新日期:2017-12-01 00:00:00
abstract:RATIONALE:Amyotrophic lateral sclerosis (ALS) is a rapidly progressing neurodegenerative disease that involves limb, axial, bulbar, and respiratory muscles. Fatigue and weakness of the respiratory muscles eventually induce respiratory insufficiency, which is one of the main causes of death in patients with ALS. In ALS ...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0000000000007899
更新日期:2017-08-01 00:00:00
abstract::To investigate the epidemiology of systemic sclerosis (SSc) in southern Europe, we assessed the incidence, prevalence, clinical spectrum, and survival of patients diagnosed with SSc in the Lugo region of northwestern Spain. Between January 1988 and December 2006, SSc was diagnosed in 78 Lugo residents according to the...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0b013e318189372f
更新日期:2008-09-01 00:00:00
abstract:RATIONALE:Pleomorphic rhabdomyosarcoma (PRMS) is a rare soft tissue malignancy which is frequently misdiagnosed and associated with metastasis to the lungs, lymph nodes, and bone marrow. Case studies are needed to improve the awareness of the disease and our understanding of it. PATIENT CONCERNS:In this study, we pres...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0000000000013648
更新日期:2018-12-01 00:00:00
abstract::[In the article "Uncompromised Treatment Efficacy in Elderly Patients With Hepatocellular Carcinoma: A Propensity Score Analysis", which appeared in Volume 93, Issue 28 of Medicine, one of the authors' affiliations, National Yang-Ming University School of Medicine, Taipei, Taiwan, was omitted. The article has since be...
journal_title:Medicine
pub_type: 已发布勘误
doi:10.1097/01.md.0000461199.07356.ca
更新日期:2015-01-01 00:00:00
abstract::Aim of this study was to determine if surface electromyography (sEMG) could provide objective data in monitoring the alteration of signal amplitude of myoelectric activity of the paraspinal muscles in the patients with acute nonspecific lower back pain (ANLBP), and to explore the correlation between sEMG data and symp...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0000000000016904
更新日期:2019-08-01 00:00:00
abstract::Impact of arterial stiffness on aortic morphology has not been well evaluated. We sought to investigate the association of brachial-ankle pulse wave velocity (baPWV) with aortic calcification and tortuosity.A total of 181 patients (65.4 ± 10.4 years, males 59.7%) who underwent computed tomographic angiography and baPW...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0000000000016802
更新日期:2019-08-01 00:00:00
abstract:RATIONALE:Paraneoplastic cerebellar degeneration (PCD) is a rare neurodegenerative syndrome associated with antibodies targeting the Purkinje cells of the cerebellum. Most cases of anti-Yo-associated PCD occur in females, with <20 cases reported in males. Herein, we report a male patient with anti-Yo-associated PCD who...
journal_title:Medicine
pub_type: 杂志文章,评审
doi:10.1097/MD.0000000000021760
更新日期:2020-08-14 00:00:00
abstract::Pyogenic sacroiliitis is exceptional in very young children. Diagnosis is difficult because clinical examination is misleading. FABER test is rarely helpful in very young children. Inflammatory syndrome is frequent. Bone scintigraphy and MRI are very sensitive for the diagnosis. Joint fluid aspiration and blood cultur...
journal_title:Medicine
pub_type: 杂志文章,评审
doi:10.1097/MD.0000000000001581
更新日期:2015-10-01 00:00:00
abstract:RATIONALE:Sodium valproate (VPA) and lamotrigine (LTG) are widely used antiepileptic drugs, disabling postural, and action tremors after using LTG with VPA were reported in 1993. However, in this study, we describe a patient in whom disabling resting-type tremor induced by 2-year use of VPA and LTG. PATIENT CONCERNS:A...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0000000000008711
更新日期:2017-11-01 00:00:00
abstract::Vedolizumab is an anti-inflammatory monoclonal antibody that exclusively targets the α4β7 integrin. We aimed to systematically review the efficacy and safety of vedolizumab for patients with inflammatory bowel diseases (IBDs). PubMed, EMBASE, and the Cochrane Library were searched up to May 2014. Randomized controlled...
journal_title:Medicine
pub_type: 杂志文章,meta分析,评审
doi:10.1097/MD.0000000000000326
更新日期:2014-12-01 00:00:00
abstract::Diabetes mellitus (DM) is prevalent in patients with pancreatic cancer and tends to improve after tumor resection. However, the glycemic response of non-pancreatic cancer patients after surgery has not been examined in detail. We aimed to investigate the changes in glucose metabolism in patients with pancreatic cancer...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0000000000010590
更新日期:2018-05-01 00:00:00
abstract::This study aimed to evaluate the characteristics of parathyroid carcinoma and to validate the diagnostic value of Tc-methoxyisobutylisonitrile (MIBI) single photon emission computed tomography/x-ray computed tomography (SPECT/CT) for differentiating between parathyroid carcinoma and hyperparathyroidism. Four consecuti...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0000000000012578
更新日期:2018-10-01 00:00:00
abstract::This study used a system evaluation method to summarize China's experience on improving the coverage of hepatitis B vaccine, especially the strategies employed to improve the uptake of timely birth dosage. Identifying successful methods and strategies will provide strong evidence for policy makers and health workers i...
journal_title:Medicine
pub_type: 杂志文章,评审
doi:10.1097/MD.0000000000003693
更新日期:2016-05-01 00:00:00
abstract::This study aimed to explore the feasible effect of ezetimibe for postprandial hyperlipidemia (PPHP).Sixty participants were included in this study. Of these, 30 subjects in the intervention group received ezetimibe, while the remaining 30 participants in the control group did not undergo ezetimibe. All patients in int...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0000000000012960
更新日期:2018-11-01 00:00:00
abstract:BACKGROUND:Raman spectroscopy could be applied to distinguish tumor from normal tissues. This meta-analysis assessed the accuracy of Raman spectroscopy in differentiating skin cancer from normal tissue. METHODS:PubMed, Embase, Cochrane Library, and CNKI were searched to identify suitable studies before Februray 4th, 2...
journal_title:Medicine
pub_type: 杂志文章,meta分析,评审
doi:10.1097/MD.0000000000012022
更新日期:2018-08-01 00:00:00
abstract::To investigate the differences in bone mineral density between patients with liver cirrhosis and healthy control, and to analyze the risk factors of hepatic osteoporosis in patients with HBV related liver cirrhosis.A total of 189 patients with liver cirrhosis and 207 health controls were enrolled. The bone mineral den...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0000000000016628
更新日期:2019-08-01 00:00:00
abstract:RATIONALE:Multiorgan/system injury was observed in severely infected coronavirus disease 2019 (COVID-19) patients, in addition to viral pneumonia. Recognizing and correcting the key and immediate dysfunctions may reduce mortality. PATIENT CONCERNS:A 66-year-old previously healthy male patient was referred to the isola...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0000000000023449
更新日期:2020-12-04 00:00:00
abstract:BACKGROUND:Traumatic brain injury (TBI) refers to head injuries that disrupt normal function of the brain. TBI commonly lead to a wide range of potential psychosocial functional deficits. Although psychosocial function after TBI is influenced by many factors, more and more evidence shows that social cognitive skills ar...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0000000000021154
更新日期:2020-07-17 00:00:00
abstract:BACKGROUND:The role of real-time elastography (RTE)-targeted biopsy in the detection and diagnosis of prostate cancer (PCa) remains controversial. METHODS:We searched Medline, Embase, and Cochrane Library from inception to July 31, 2017 and used the Quality Assessment of Diagnostic Accuracy Studies (QUADAS-2) tool to ...
journal_title:Medicine
pub_type: 杂志文章,meta分析,评审
doi:10.1097/MD.0000000000010220
更新日期:2018-03-01 00:00:00
abstract::Cardiovascular disease (CVD) is a common cause of death in patients with chronic kidney disease (CKD). Aortic and mitral valve calcification (AVC and MVC, respectively) are critical indicators of CVD and all-cause mortality in CKD patients.We conducted a single center retrospective study of Chinese inpatients with CKD...
journal_title:Medicine
pub_type: 杂志文章
doi:10.1097/MD.0000000000009804
更新日期:2018-02-01 00:00:00